Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a usually unilateral facial port-wine stain, ipsilateral leptomeningeal angiomatosis, glaucoma, epileptic seizures that are difficult to control, and progressive cerebral dysfunction. A 41-year-old woman presented with headache, nausea, vomiting, and a speech disorder. On examination a lesion on the right side of her face consistent with hemangioma was noted, as well as a high fever, hyperglycemia, and tonsillitis. Electroencephalography revealed a paroxysmal abnormality in the right frontotemporal region, and contrast-enhanced cranial magnetic resonance imaging revealed vascularization and dural contrast enhancement in the right cerebral hemisphere. The patient was diagnosed with SWS and discharged with acetylsalicylic acid (ASA) and valproic acid therapy, but returned 2 weeks later with tonic-clonic status epilepticus. The seizures were controlled with an infusion of diazepam in the intensive care unit and the dosage of valproic acid was increased. Aripiprazole was initiated for visual and auditory hallucinations. The patient was discharged after 10 days with a normal neurological examination and follow-up has not revealed any seizure or sequel findings with the ASA and valproic acid therapy.